Department of Scienze dell'Emergenza, Anestesiologiche e della Rianimazione, IRCCS Fondazione Policlinico Universitario A. Gemelli, Rome, Italy.
BMC Anesthesiol. 2023 Mar 28;23(1):94. doi: 10.1186/s12871-023-02066-4.
Atypical Haemolytic Uremic Syndrome is an acute life-threatening condition, characterized by the clinical triad of microangiopathic hemolytic anaemia, thrombocytopenia, kidney injury. Management of pregnants affected by Atypical Haemolytic Uremic Syndrome can be a serious concern for obstetric anesthesiologist in the delivery room and in the intensive care unit.
A 35-year-old primigravida with a monochorionic diamniotic twin pregnancy, presented with an acute haemorrhage due to retained placenta after elective caesarean section and underwent surgical exploration. In the postoperative period, the patient progressively developed hypoxemic respiratory failure and, later on, anaemia, severe thrombocytopenia, and acute kidney injury. A timely diagnosis of Atypical Haemolytic Uremic Syndrome was made. Non-invasive ventilation and high-flow nasal cannula oxygen therapy sessions were initially required. Hypertensive crisis and fluid overload were aggressively treated with a combination of beta and alpha adrenergic blockers (labetalol 0,3 mg/kg/h by continuous intravenous infusion for the first 24 hours, bisoprolol 2,5 mg twice daily for the first 48 hours, doxazosin 2 mg twice daily), central sympatholytics (methyldopa 250 mg twice daily for the first 72 hours, transdermal clonidine 5 mg by the third day), diuretics (furosemide 20 mg three times daily), calcium antagonists (amlodipine 5 mg twice daily). Eculizumab 900 mg was administered via intravenous infusion once per week, attaining hematological and renal remissions. The patient also received several blood transfusion units and anti- meningococcal B, anti-pneumococcal, anti-haemophilus influenzae type B vaccination. Her clinical condition progressively improved, and she was finally discharged from intensive care unit 5 days after admission.
The clinical course of this report underlines how crucial it is for the obstetric anaesthesiologist to promptly identify Atypical Haemolytic Uremic Syndrome, since early initiation of eculizumab, together with supportive therapy, has a direct effect on patient outcome.
非典型溶血尿毒综合征是一种危及生命的急性疾病，其特征是微血管性溶血性贫血、血小板减少和肾损伤的临床三联征。产科麻醉师在产房和重症监护病房中对受非典型溶血尿毒综合征影响的孕妇进行管理可能是一个严重的问题。
一位 35 岁的初产妇，怀单绒毛膜双羊膜囊双胞胎，选择性剖宫产术后因胎盘滞留导致急性出血，并进行了手术探查。术后，患者逐渐出现低氧性呼吸衰竭，随后出现贫血、严重血小板减少和急性肾损伤。及时诊断出非典型溶血尿毒综合征。最初需要进行无创通气和高流量鼻导管吸氧治疗。使用β和α肾上腺素能阻滞剂（拉贝洛尔 0.3mg/kg/h 持续静脉输注 24 小时，比索洛尔 2.5mg 每日两次 48 小时，多沙唑嗪 2mg 每日两次）联合治疗高血压危象和液体超负荷，使用中枢交感神经抑制剂（甲基多巴 250mg 每日两次 72 小时，透皮可乐定 5mg 第 3 天）、利尿剂（呋塞米 20mg 每日三次）、钙拮抗剂（氨氯地平 5mg 每日两次）。每周通过静脉输注给予艾库珠单抗 900mg，达到血液学和肾脏缓解。患者还接受了几次输血单位和脑膜炎球菌 B 型、肺炎球菌、流感嗜血杆菌 B 型疫苗接种。她的临床状况逐渐改善，最终在入院后 5 天从重症监护病房出院。
本报告的临床过程强调了产科麻醉师及时识别非典型溶血尿毒综合征的重要性，因为早期使用艾库珠单抗联合支持治疗对患者的预后有直接影响。
